Scleroderma

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Scleroderma is a chronic disorder characterized by diffuse fibrosis of skin and internal organs. The cause of scleroderma is not known.

Autoimmunity, fibroblast dysregulation, graft-versus-host disease, fetal lymphocytes retained in maternal circulation and occupational exposure to silica have been made responsible for the outcome of scleroderma.

Types of Scleroderma

Scleroderma may be localized or diffuse.
scleroderma

Limited Scleroderma
It is prevalent in 80% of patients and is also known as CREST* syndrome, in which hardening of skin is limited to the face and hands.

*CREST= calcinosis cutis, Raynaud’s phenomenon, esophageal motility disorder, sclerodactyly, and telangiestasia

Diffuse Scleroderma
Its prevalence is about 20% in patients. In diffuse form of scleroderma, skin changes occur in the trunk and proximal extremities. Tendon friction rubs especially over wrists, ankles and knees.

Clinical Features

Musculo Skeletal
Polyarthralgia
Raynaud’s phenomenon

Gastrointestinal tract
Dysphagia
Gastroesophageal Reflux disease
Large mouthed diverticulli (jejunum, ileum, colon)

Pulmonary
Pulmonary Hypertension
Pulmonary firbrosis

Cardiac Abnormalities
Pericarditis
Heart block
Myocardial fibrosis

Renal Problems
Scleroderma renal crisis

Laboratory Findings

Microangiopathic hemolytic anemia in patients with severe renal disease.
Antinuclear Antibody tests are always positve.
Anticentomere antibody is seen in 50% of those with CREST syndrome.
Anti-Scl-70 antibodies may show a poor prognosis, with a high likelihood of serious internal organ involvement (e.g interstitial lung diseases).
Anti-RNAP antibodies are associated with diffuse skin changes.

Treatment

  • Raynaud’s syndrome may respond to calcium channel’s blockers. E.g. Nifedipine 30-120mg/day or Losartan 50mg/day.
  • Intravenous Iloprost causes vasodilation and platelate inhibition.
  • Tetracyclines 500mg four times daily inhibit the growth of bacteria
  • Captopril 37.5-75mg/d in three divided doses for the treament of scleroderma renal crisis.
  • Cyclophosphamide is used to improve severe interstitial lung disease
  • Bosentan improves pulmonary hypertension.
  • NSAIDS and Steroids are used for active musculoskeletal disease.

Photo courtesy of Leith C Jones at en.wikipedia

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The writer enjoys medical education and has special interest in community medicine.